Last reviewed: 25 Dec 2021
Last updated: 04 Feb 2021



History and exam

Other diagnostic factors

  • recent sore throat or scarlet fever
  • chest pain
  • shortness of breath
  • palpitations
  • heart murmur
  • pericardial rub
  • signs of cardiac failure
  • swollen joints
  • restlessness
  • clumsiness
  • emotional lability and personality changes
  • jerky, uncoordinated choreiform movements
  • inability to maintain protrusion of the tongue
  • milkmaid's grip
  • spooning sign
  • pronator sign
  • erythema marginatum
  • subcutaneous nodules
  • pregnancy or taking oral contraceptive pill

Risk factors

  • poverty
  • overcrowded living quarters
  • family history of rheumatic fever
  • D8/17 B cell antigen positivity
  • HLA association
  • genetic susceptibility
  • indigenous populations; Aboriginal Australian, Asian, and Pacific Islanders

Diagnostic investigations

1st investigations to order

  • erythrocyte sedimentation rate (ESR)
  • CRP
  • WBC count
  • blood cultures
  • electrocardiogram
  • chest x-ray
  • echocardiogram
  • throat culture
  • rapid antigen test for group A streptococci
  • anti-streptococcal serology
  • rapid molecular test

Treatment algorithm



Rachel Webb, MbChB, MPH & THM, FRACP

Senior Lecturer in Paediatrics

University of Auckland

Paediatric Infectious Diseases Specialist

Starship Children's Hospital and Middlemore Hospital


New Zealand


RW declares that she has no competing interests; she is an active researcher and clinician in acute rheumatic fever/rheumatic heart disease and is a co-investigator on a (non-industry) grant funded by the Health Research Council of New Zealand and gives educational talks and has prepared manuscripts on rheumatic fever solely in capacity as a University of Auckland academic and Paediatric Infectious Diseases Specialist.


Dr Rachel Webb would like to gratefully acknowledge Dr Andrew C. Steer and Dr Jonathan Carapetis, previous contributors to this topic.


ACS and JC declare that they have no competing interests.

Peer reviewers

Salah Zaher, MD

Professor of Pediatrics

Division of Pediatric Cardiology

Faculty of Medicine

University of Alexandria


El Shatby Children's Hospital




SZ declares that she has no competing interests.

Nigel Wilson, FRACP

Paediatric Cardiologist/Interventional Cardiologist

Paediatric and Congenital Cardiac Services

Green Lane Clinical Services

Starship Children's Hospital


New Zealand


NW declares that he has no competing interests.

Andrea Summer, MD

Assistant Professor of Pediatrics

Medical University of South Carolina




AS declares that she has no competing interests.

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